Yu W Haung, Cuervo Ana Maria, Kumar Asok, Peterhoff Corrinne M, Schmidt Stephen D, Lee Ju-Hyun, Mohan Panaiyur S, Mercken Marc, Farmery Mark R, Tjernberg Lars O, Jiang Ying, Duff Karen, Uchiyama Yasuo, Näslund Jan, Mathews Paul M, Cataldo Anne M, Nixon Ralph A
Center for Dementia Research, Nathan Kline Institute, Orangeburg, NY 10962, USA.
J Cell Biol. 2005 Oct 10;171(1):87-98. doi: 10.1083/jcb.200505082. Epub 2005 Oct 3.
Macroautophagy, which is a lysosomal pathway for the turnover of organelles and long-lived proteins, is a key determinant of cell survival and longevity. In this study, we show that neuronal macroautophagy is induced early in Alzheimer's disease (AD) and before beta-amyloid (Abeta) deposits extracellularly in the presenilin (PS) 1/Abeta precursor protein (APP) mouse model of beta-amyloidosis. Subsequently, autophagosomes and late autophagic vacuoles (AVs) accumulate markedly in dystrophic dendrites, implying an impaired maturation of AVs to lysosomes. Immunolabeling identifies AVs in the brain as a major reservoir of intracellular Abeta. Purified AVs contain APP and beta-cleaved APP and are highly enriched in PS1, nicastrin, and PS-dependent gamma-secretase activity. Inducing or inhibiting macroautophagy in neuronal and nonneuronal cells by modulating mammalian target of rapamycin kinase elicits parallel changes in AV proliferation and Abeta production. Our results, therefore, link beta-amyloidogenic and cell survival pathways through macroautophagy, which is activated and is abnormal in AD.
巨自噬是一种用于细胞器和长寿蛋白周转的溶酶体途径,是细胞存活和寿命的关键决定因素。在本研究中,我们发现,在早老素(PS)1/β-淀粉样前体蛋白(APP)β-淀粉样变小鼠模型中,神经元巨自噬在阿尔茨海默病(AD)早期且在β-淀粉样蛋白(Aβ)在细胞外沉积之前就被诱导。随后,自噬体和晚期自噬泡(AVs)在营养不良的树突中显著积累,这意味着AVs向溶酶体的成熟受损。免疫标记将大脑中的AVs鉴定为细胞内Aβ的主要储存库。纯化的AVs含有APP和β-裂解的APP,并且高度富集PS1、尼卡斯特林和PS依赖的γ-分泌酶活性。通过调节雷帕霉素激酶的哺乳动物靶点来诱导或抑制神经元和非神经元细胞中的巨自噬,会引起AVs增殖和Aβ产生的平行变化。因此,我们的结果通过巨自噬将β-淀粉样生成和细胞存活途径联系起来,巨自噬在AD中被激活且异常。